Focal segmental glomerulosclerosis in children with idiopathic nephrotic syndrome. A report of the Southwest Pediatric Nephrology Study Group1

Focal segmental glomerulosclerosis in children with idiopathic nephrotic syndrome. A report of the Southwest Pediatric Nephrology Study Group. Clinicopathologic correlations were examined in 75 children with focal segmental glomerulosclerosis (FSGS) associated with idiopathic nephrotic syndrome. The biopsy specimens of all patients were examined by electron microscopy (69 patients) or immunofluorescence microscopy (67 patients) in addition to light microscopy. Fifty-three patients (group A) had FSGS diagnosed on their first biopsy; 22 patients (group B) had one to three previous biopsies showing minimal glomerular changes or mesangial hypercellularity prior to the demonstration of FSGS on a subsequent biopsy. Considerable homogeneity between the diagnostic biopsy features in the two groups was evident. Diffuse mesangial hypercellularity and IgM deposition were found in a similar percentage of each group, but these features did not correlate with each other. To date, the mean duration of follow-up for the entire group has been 57 months (range, 7 to 217 months): 21% have developed ESRD, 23% have a decreased GFR but not ESRD, 37% have persistent proteinuria only, 11% are in remission, and 8% have been lost to follow-up. No morphologic or clinical features have been predictive of outcome during this relatively short period of foliowup. The frequency of chronic renal failure and ESRD has been similar in groups A and B. These data suggest that the clinical outcome in children with FSGS is poor in many patients, whether the diagnosis is established on an initial or subsequent renal biopsy specimen. Glomerulosclerose focale et segmentaire chez des enfants atteints de syndrome nephrotique idiopathique. Un rapport du Southwest Pediatric Nephrology Study Group. Des correlations anatomo-cliniques ont ete examinees chez 75 enfants atteints de glomerulosclerose focale et segmentaire (FSGS) associee a un syndrome nephrotique. Les specimens biopsies de tous les malades ont ete etudiees en microscopie electronique (69 malades) ou en microscopie avec immunofluorescence (67 malades) en plus de la microscopie optique. Cinquante-trois malades (groupe A) avaient une FSGS diagnostiquee a la premiere biopsie; 22 malades (groupe B) avaient eu une a trois biopsies anterieures indiquant des lesions glomerulaires minimes ou une hypercellularite mesangiale avant la demonstration de la FSGS par une biopsie ulterieure. Une homogeneite considerable entre les caracteristiques de la biopsie diagnostique etait evidente dans les deux groupes. Une hypercellularite mesangiale diffuse et des depots d'IgM ont ete trouves en proportion identique dans chaque groupe, mais ces caracteristiques n'etaient pas correlees entre elles. A ce jour, la duree moyenne du suivi du groupe entier a ete de 57 mois (extremes 7 a 217 mois): 21% ont developpe une ESRD, 23% ont une GFR diminuee mais pas d'ESRD, 37% ont une proteinurie persistante seulement, 11% sont en remission, et 8% ont ete perdus de vue. Aucune caracteristique morphologique ou clinique n'a permis de predire le devenir pendant cette periode de suivi relativement courte. La frequence d'une insuffisance renale chronique et d'une ESRD etait identique dans les groupes A et B. Ces donnees suggerent que le devenir clinique de malades atteints de FSGS est sombre chez beaucoup de malades, que le diagnostic soit etabli sur un fragment de biopsie renale initiale ou ulterieure.

[1]  J. Velosa,et al.  Focal sclerosing glomerulonephropathy: a clinicopathologic study. , 1975, Mayo Clinic proceedings.

[2]  R. Glassock,et al.  Nehprotic syndrome with glomerular mesangial IgM deposits. , 1978, Laboratory investigation; a journal of technical methods and pathology.

[3]  H. Bachmann,et al.  Association of minimal change nephrotic syndrome (MCNS) with HLA-B8 an B13. , 1981, Clinical nephrology.

[4]  A. Spitzer,et al.  The significance of focal sclerotic lesions of glomeruli in children. , 1976, The Journal of pediatrics.

[5]  S. Poucell,et al.  Focal segmental glomerulosclerosis with idiopathic nephrotic syndrome: three types of clinical response. , 1982, The Journal of pediatrics.

[6]  E. Kohaut,et al.  The significance of focal glomerular sclerosis in children who have nephrotic syndrome. , 1976, American journal of clinical pathology.

[7]  C. Brown,et al.  Focal segmental glomerulosclerosis with rapid decline in renal function ("malignant FSGS"). , 1978, Clinical nephrology.

[8]  J. Churg,et al.  Intraglomerular fibrin, platelet aggregation, and subendothelial deposits in lipoid nephrosis. , 1970, The Journal of clinical investigation.

[9]  L. Hyman,et al.  Focal sclerosing glomerulonephropathy with hyalinosis , 1974 .

[10]  C. Chantler,et al.  The long-term prognosis of patients with focal segmental glomerulosclerosis. , 1978, Clinical nephrology.

[11]  R. Habib,et al.  The primary nephrotic syndrome of childhood. Classification and clinicopathologic study of 406 cases. , 1971, Pathology annual.

[12]  E. Glasgow,et al.  Focal glomerulosclerosis in childhood. , 1973, Perspectives in nephrology and hypertension.

[13]  S. O'regan,et al.  Primary nephrosis in childhood associated with focal glomerular sclerosis: is long-term prognosis that severe? , 1981, Kidney international.

[14]  A. Richman,et al.  Endothelial and platelet reactions in the idiopathic nephrotic syndrome: an ultrastructural study. , 1982, Human pathology.

[15]  J. Churg,et al.  Pathology of the nephrotic syndrome in children: a report for the International Study of Kidney Disease in Children. , 1970, Lancet.

[16]  W. Grupe.  Relapsing nephrotic syndrome in childhood. , 1979, Kidney international.

[17]  R. Habib.  Focal glomerular sclerosis , 1973 .

[18]  L. Hyman,et al.  Focal sclerosing glomerulonephropathy with segmental hyalinosis. A clinicopathologic analysis. , 1973, Laboratory investigation; a journal of technical methods and pathology.

[19]  D. Woodrow,et al.  Focal and segmental hyalinosis. , 1975, Advances in nephrology from the Necker Hospital.

[20]  M. Schoeneman,et al.  The natural history of focal segmental glomerulosclerosis with and without mesangial hypercellularity in children. , 1978, Clinical nephrology.

[21]  F. Scheler,et al.  Minimal change lesion with nephrotic syndrome and focal glomerular sclerosis. (Variations of minimal proliferative intercapillary glomerulonephritis with the nephrotic syndrome). , 1974, Clinical nephrology.

[22]  G. Schwartz,et al.  A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine , 2004 .

[23]  D. Ellis,et al.  Focal glomerulosclerosis in children: correlation of histology with prognosis. , 1978, The Journal of pediatrics.

[24]  R. R. Robinson,et al.  FOCAL GLOMERULAR SCLEROSIS: CONTRASTING CLINICAL PATTERNS IN CHILDREN AND ADULTS , 1976, Medicine.

[25]  A. Nicastri,et al.  Long-term evaluation of children with nephrotic syndrome and focal segmental glomerular sclerosis. , 1983, Nephron.

[26]  M. Legrain,et al.  Focal glomerulosclerosis: natural history and treatment. A report of 70 cases. , 1978, Nephron.

[27]  E. Kohaut,et al.  Focal glomerulosclerosis. Extent of involvement related to steroid resistance. , 1981, American journal of clinical pathology.